Department of Diabetes & Endocrinology, Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Leicester, UK.
Clin Endocrinol (Oxf). 2010 Jul;73(1):18-21. doi: 10.1111/j.1365-2265.2009.03765.x. Epub 2009 Dec 18.
Autoimmune hypophysitis is a rare chronic inflammatory condition of the pituitary gland which typically presents with hypopituitarism and a pituitary mass. Cases involving anterior pituitary alone (65%) are six times more common in women, typically presenting during pregnancy or postpartum (57%). Anterior and posterior pituitary involvement (25%) are twice as common in women, and neurohypophysis alone (10%) occurs equally in both sexes. It has a prevalence of around 5 per million, an annual incidence of 1 in 7 to 9 million and in our experience represents the known or suspected cause of 0.5% of cases of hypopituitarism, <1% of pituitary masses and 2% of nonfunctioning macro lesions presenting to an endocrine clinic. However, 'missed' cases of autoimmune hypophysitis may be the aetiology of some other unexplained cases of hypopituitarism. Clinically, headache and visual disturbance are common. Anterior hypopituitarism shows a characteristic but atypical pattern of deficiency of ACTH followed by TSH, gonadotrophins and prolactin deficiency or hyperprolactinaemia. Eighteen percent of cases have evidence of another autoimmune condition. On magnetic resonance imaging (MRI), autoimmune hypophysitis is typically symmetrical and homogeneous with thickened but undisplaced stalk in contrast to typical findings with pituitary tumours. Ultimately, the histological diagnosis of autoimmune hypophysitis can only be confirmed by surgery but a presumptive diagnosis can often be made on the basis of a combination of context and clinical features, and pituitary biopsy is not always clinically necessary for effective clinical management of the patient.
自身免疫性垂体炎是一种罕见的慢性垂体炎症性疾病,通常表现为垂体功能减退和垂体肿块。单纯累及垂体前叶(65%)的病例在女性中更为常见,通常发生在妊娠或产后(57%)。累及垂体前叶和后叶(25%)的病例在女性中更为常见,而单纯累及神经垂体(10%)在两性中发生的概率相同。其患病率约为每百万分之五,年发病率为每 700 万至 900 万分之一,根据我们的经验,它是已知或疑似导致 0.5%的垂体功能减退症、<1%的垂体肿块和 2%的无功能大病变的原因,这些病变出现在内分泌科诊所。然而,“漏诊”的自身免疫性垂体炎可能是某些其他不明原因的垂体功能减退症的病因。临床上,头痛和视力障碍很常见。垂体前叶功能减退症表现为一种典型但非典型的 ACTH 缺乏模式,随后是 TSH、促性腺激素和催乳素缺乏或高催乳素血症。18%的病例有其他自身免疫性疾病的证据。在磁共振成像(MRI)上,自身免疫性垂体炎通常是对称和均匀的,伴有增厚但未移位的柄,与典型的垂体瘤表现不同。最终,自身免疫性垂体炎的组织学诊断只能通过手术确认,但在基于上下文和临床特征的基础上,通常可以做出推定诊断,而且并非所有情况下对患者进行有效的临床管理都需要进行垂体活检。